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Macrophage activation syndrome (MAS) is a form of secondary hemophagocytic lymphohistiocytosis and is a rapidly progressive, life-threatening complication of adult-onset Still disease (AOSD). An an...

We report a rare presentation of adult-onset Still disease (AoSD) with flagellate dermatosis and unknown trigger. Atypical skin findings have been increasingly reported for AoSD and may be associated with worse prognosis and systemic complications. Increased awareness of nonclassic skin findings in AoSD may lead to earlier diagnosis and treatment.

Adult-onset Still Disease (AOSD) is a rare rheumatologic condition with unrecognized etiology. Spiking fevers, joint involvement (arthralgia or arthritis), rashes, lymphadenopathy, abnormal liver function test data, and leukocytosis are the main features of this disease. Yamaguchi’s criteria with the sensitivity and specificity of 96.2% and 92.1%, respectively, is the most beneficial tool for the diagnosis of AOSD, as a rare disease. The uncommon features of the still disease remain confusing for clinicians. about the present study reported a 27-year-old patient who was referred to our hospital with an unknown diagnosis, because of the rare features of Still disease. The explored characteristics of rashes, fever, and para-clinical data, as well as laboratory and imaging data, are described in detail; such features have led to long hospitalization to confirm the diagnosis in this patient. The positive family history of the rheumatologic disease and dramatic response to low dose corticosteroids were other outstanding features of our case. This report highlighted the necessity of conducting randomized clinical trials to address the management of AOSD. Besides, the present study signified the need for providing further awareness among clinicians to prevent long hospitalization.

Adult-onset Still disease (AOSD) is a rare autoinflammatory condition. The presence of an evanescent, salmon-pink, nonpruritic rash is one of the major diagnostic criteria for the disease. The rash occurs with fever and subsides with defervescence. The presence of dyskeratotic keratinocytes in the upper one-third layer of the epidermis is a distinctive histopathological feature of persistent pruritic lesions associated with AOSD. Here, we report 2 cases of AOSD characterized by persistent pruritic lesions resembling those observed in dermatomyositis. Identifying the clinical and histopathological manifestation of the cutaneous lesions is essential for the early diagnosis of AOSD and for differentiating this condition from those presenting with dyskeratotic cells in the epidermis.

Pseudoprogression is a unique, uncommon phenomenon that often hints at good prognosis. To date, there has been no report of complete remission after pseudoprogression. Here, we report successful treatment of metastatic renal clear cell cancer, using anti-PD-1 antibodies combined with autologous RetroNectin-activated cytokine-induced killer cells, in two patients who were failed to multitargeted kinase inhibitors. After early pseudoprogression, complete remission was achieved. At present, one patient has stopped therapy for about 1.5 years and is still disease free.

A 19-year-old Caucasian female with adult-onset Still disease (AOSD) presented for evaluation of an acute clinical decompensation and atypical annular papules and plaques with purpura on the lower extremities. A punch biopsy demonstrated histiocytes with engulfed degenerated erythrocytes and lymphocytes, consistent with hemophagocytic lymphohistiocytosis (HLH). HLH, clinically referred to as macrophage activation syndrome, is a rare complication of AOSD and is life-threatening. Relevant clinical, laboratory, and histologic features of this diagnosis are reviewed.

utoinflammatory diseases are characterized by recurrent systemic inflammation due to dysfunction of the innate immune system, and they are originally the hereditary recurrent fever syndromes that develop in early childhood. Many cases are thus diagnosed in the pediatric field, but there are many cases that are not diagnosed until adulthood, including adult-onset cases. Accordingly, not only pediatricians but also rheumatologists and primary care physicians play a major role in the diagnosis and treatment of autoinflammatory diseases. Broad non-hereditary autoinflammatory diseases such as adult-onset Still disease and Behcet's disease are also often encountered in routine internal medicine practice. With recent developments in genetic testing, the conditions of patients who have been diagnosed and treated as having fevers of unknown etiology have been increasingly classified as autoinflammatory diseases. Regarding treatment in Japan, biological agents (such as canakinumab) that inhibit IL-1β are now administered to patients with autoinflammatory diseases. In this review, we introduce an approach from rheumatologists based on diagnoses and treatment of ‘classical” autoinflammatory diseases in adults,focusing on familial Mediterranean fever, which has a high frequency in adults., Immunological Medicine, 41(4), pp.177-180; 2019

Autoinflammatory diseases are characterized by innate immunity abnormalities. In autoinflammatory diseases (AID), inflammatory blood biomarkers are elevated during crisis without infection and usually without autoantibodies. The first 4 described AID were familial Mediterranean fever, cryopyrin-associated periodic fever syndrome (CAPS) or NLRP3-associated autoinflammatory disease (NRLP3-AID), mevalonate kinase deficiency (MKD) and TNFRSF1A-receptor associated periodic fever syndrome (TRAPS). Since their description 20 years ago, and with the progresses of genetic analysis, many new diseases have been discovered; some with recurrent fever, others with predominant cutaneous symptoms or even immune deficiency. After describing the 4 historical recurrent fevers, some polygenic inflammatory diseases will also be shortly described such as Still disease and periodic fever with adenitis, pharyngitis and aphtous (PFAPA) syndrome. To better explore AID, some key anamnesis features are crucial such as the family tree, the age at onset, crisis length and organs involved in the clinical symptoms. An acute phase response is mandatory in crisis.

Approximately 30% to 40% of all patients with adult-onset Still disease (AOSD) experience relapses, sometimes presenting as chronic damage, and these events can subsequently increase the morbidity and mortality in patients with AOSD. However, few studies are investigating the factors related to relapse in such patients. Therefore, this study aimed to explore the risk factors associated with relapse of AOSD. This cohort study enrolled 112 AOSD patients who satisfied the Yamaguchi criteria and obtained available data from Chonnam National University Hospital. The demographic, clinical, and laboratory data as well as treatment history of the patients from January 2008 to December 2019 were retrospectively reviewed. Relapse events were defined as the presence of one or more recurrent events. Multivariate logistic regression analysis was performed to investigate the possible risk factors for relapse. During a mean follow-up of 103.3 months, 47 of 112 patients (41.9%) developed a relapse. According to the results of multivariate logistic regression analysis, arthritis (odds ratio [OR] = 19.530, 95% confidence interval [CI]: 5.047–75.582, P

Schnitzler Syndrome is a rare acquired auto-inflammatory syndrome defined by an urticarial eruption and a monoclonal gammopathy, mainly of the IgM kappa isotype. It shares many clinical and biological features with other autoinflammatory disorders such as NLRP3-auto-inflammatory disorders (NLRP3-AID, formerly cryopyrin associated periodic syndromes or CAPS) or adult-onset Still disease (AOSD). Hence, recurrent fever, urticarial rash with a neutrophilic infiltrate on skin biopsy (i.e. neutrophilic urticarial dermatosis or NUD) and a significant elevation of blood inflammation markers are commonly found in Schnitzler Syndrome as well as in NLRP3-AID or AOSD. IL-1s plays a crucial role in the pathogenesis and explains the clinical symptoms of Schnitzler Syndrome. This is emphasized by the spectacular effectiveness of IL-1 blocking therapies, especially anakinra. IL-1 blocking therapies are efficient on the inflammation-linked symptoms but not on the monoclonal component. The evolution is chronic and about 15-20% of patients may develop lymphoproliferative disease, in particular Waldenstrom disease, a proportion similar to patients with IgM monoclonal gammopathy of undetermined significance, and more rarely AA-amyloidosis.

Macrophage activation syndrome (MAS) is a form of secondary hemophagocytic lymphohistiocytosis (HLH) and is a life-threatening complication of adult-onset Still disease. MAS has been usually treated with high-dose glucocorticoid with additional immunosuppressive agents, such as cyclosporine. Etoposide has been used for the treatment of severe refractory MAS based on the successful results of HLH-2004 protocol in patients with mostly primary form of HLH. We herein describe a case of severe refractory MAS secondary to adult-onset Still disease in an elderly woman that inadequately responded to etoposide but remarkably responded to additional tocilizumab. Furthermore, short-term tocilizumab led her into remission and enabled tapering off glucocorticoids after 15 months. Tocilizumab may be effective for the treatment of refractory HLH after the failure of the etoposide-containing induction regimen.

Hemophagocytic lymphohistiocytosis (HLH) represents a potentially life-threatening episode of overwhelming inflammation characterized by activation of T lymphocytes, hemophagocytic macrophages, and marked hyperferritinemia1,2. Historically, HLH has been classified as having a primary, genetic cause (familial HLH) or occurring secondary to an identified trigger including various infections, malignancies, or rheumatic diseases. When occurring in the setting of inflammatory or autoimmune disease, rheumatologists call secondary HLH the macrophage activation syndrome (MAS), and it is reflected as MAS-HLH in the updated Histiocyte Society nomenclature3. Episodes of MAS-HLH have been reported in nearly all rheumatic diseases, but are most commonly recognized in patients with systemic juvenile idiopathic arthritis (sJIA) or adult-onset Still disease (AOSD), systemic lupus erythematosus (SLE), Kawasaki disease, and certain monogenic autoinflammatory disorders4,5. In contrast, the inflammatory myopathies including dermatomyositis (DM) and polymyositis (PM) have typically been considered low-risk for development of MAS-HLH. In the current issue of The Journal , Liang and colleagues6 present a large series of patients with adult idiopathic inflammatory myopathies that challenges this conventional wisdom, and suggest that MAS-HLH may represent an underappreciated cause of mortality in these disorders. In this report, the authors identified 424 adult patients admitted to their institution over an 8-year period with DM, PM, or clinically amyopathic dermatomyositis (CADM)6. Interestingly, they found that 18 (4.2%) satisfied the 2004 diagnostic criteria for HLH7. … Address correspondence to Dr. G.S. Schulert, Division of Rheumatology, Cincinnati Children’s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45208 USA. Email: Grant.schulert{at}cchmc.org.

Cardiac involvement in Adult-onset Still’s Disease (AOSD) usually manifests as a pericardial disease, that has a benign course. The myocarditis is a rare complication with 7% of prevalence. The diagnosis of AOSD is based in the Yamaguchi or Fautrel criteria. The treatment with steroids, metrotexate is the first and second therapeutic lines, respectively, the combination is effective in 70% of cases. We report one case of AOSD with unusual presentation of double cardiac commitment, Myopericarditis. El compromiso cardíaco en la enfermedad de Still del adulto (ESA) usualmente se manifiesta como enfermedad pericárdica, la cual, generalmente, tiene un curso benigno. La miocarditis es una complicación infrecuente con una prevalencia del 7%. El diagnóstico de ESA se basa en los sets de criterios de Yamaguchi o Fautrel. El tratamiento con corticosteroides y metrotexato es la primera y segunda línea terapéutica, respectivamente, que combinados son efectivos en 70% de los casos. Reportamos un caso de ESA de presentación inusual por el compromiso tanto miocárdico como pericárdico.

Adult-onset Still's disease (AOSD) is a rare autoimmune disease of unknown etiology with systemic inflammatory manifestations consisting of a triad of fevers, evanescent skin rash, and arthritis. Although steroids are the first line of therapy, about 20%-30% of patients are refractory, intolerant, and or relapse during tapering or upon discontinuation of steroids. There are no clinical guidelines in treating such patients and treatment in these patients is challenging. Previously used biological agents have limited efficacy and hence there is a need for new therapies. Tocilizumab (TCZ), an interleukin (IL)-6 receptor antibody has been used with a clinical benefit and has shown to decrease the dose of steroids in patients with adult-onset still disease. The aim of this case report is to highlight the use of tocilizumab in relapsing and steroid intolerant cases of AOSD. The use of this drug in patients with AOSD is currently off-label. Randomized control studies can provide additional information that offers better visibility in treating AOSD patients who are steroid-resistant or intolerant. The rarity of disease possesses additional challenges in conducting these studies.

Adult Still's disease (ASD) is an inflammatory disorder with an unclear etiology. It is a rare disease that was first described more than a century ago. Its common characteristics are daily fevers, arthritis, and skin rash. ASD is diagnosed after excluding infections, malignancies, and connective tissue diseases. It has a female predominance. Classic skin rash and high serum ferritin levels are commonly associated with this condition and help in the diagnosis. Due to a lack of pathognomic clinical and laboratory features, a valid diagnostic criterion, the Yamaguchi criteria, is generally used for the diagnosis. The disease has a good prognosis with appropriate treatment. We present the case of a young gentleman who presented with fever, anemia, and leucocytosis; however, his serum ferritin levels were normal, and there was no typical salmon-colored skin rash. Hyperferritinemia developed later in the disease course, leading to a diagnosis of ASD.

Juvenile idiopathic arthritis (JIA) constitutes a group of arthritis of unknown origin that begins before the age of 16 years. Still´s disease is the systemic form of this condition. Its clinical presentation is marked by fever, rash and sometimes joint pain, in the absence of evidence of another aetiology of the fever. We present the cases of two boys aged 4 and 10 years admitted for fever, with a cerebral origin for the first and no infectious site for the second. Fever persisted after antimalarial treatment and adequate antibiotics. Ferritinaemia, elevated sedimentation rate, lactate dehydrogenase (LDH), triglycerides, and increased serum transaminases, all in the absence of evidence of other inflammatory or malignant diseases were suggestive of Still's disease. Both children received a corticosteroid therapy with progressive dose reduction associated to methotrexate during treatment. Fever disappeared within a few hours after initiation of corticosteroid therapy, with considerable improvement in clinical state. To the best of our knowledge, these cases are among the rare cases of childhood Still disease reported in sub-Saharan Africa. These cases highlight the importance of investigating non-infectious causes of persistent fever in children, in a context of infectious disease endemicity.

BACKGROUND: Adult-onset still disease (AOSD) and hemophagocytic syndrome (HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis. CASE SUMMARY: A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage. However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids; further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate. CONCLUSION: For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death.

Rheumatic diseases are various painful conditions that affect joints, bones, cartilage, tendons, ligaments, and muscles. Arthritis is a typical condition of rheumatic disease. Although rheumatoid arthritis is a representative rheumatic disease, various diseases other than rheumatoid arthritis can also affect joints, and differential diagnosis of rheumatic diseases is often difficult owing to the similar clinical manifestations. However, accurate diagnosis is crucial for an appropriate treatment strategy. The utility of fluorine 18 fluorodeoxyglucose (FDG) PET/CT has been established, and it is widely used for assessing malignancies. In addition to accumulating in tumor cells, FDG also accumulates in inflammatory tissue, allowing FDG PET/CT to demonstrate arthritis. PET/CT allows evaluation of whole-body articular and extra-articular lesions in one examination, representing a key advantage over US and MRI, which allow assessment of only a few regions because of their limited field of view. Although FDG PET/CT is sensitive for detecting inflammatory lesions, the uptake itself is nonspecific; therefore, knowledge of characteristic uptake patterns is necessary to narrow the differential diagnosis in rheumatic disease. Furthermore, pathognomonic extra-articular findings such as vasculitis, skin lesions, lymphadenopathy, and chondritis play an important role in achieving accurate diagnosis. The authors present the FDG PET/CT appearances of (a) rheumatoid arthritis and allied disorders (polymyalgia rheumatica, remitting seronegative symmetrical synovitis with pitting edema, adult-onset Still disease), (b) spondyloarthritis (ankylosing spondylitis, psoriatic arthritis, reactive arthritis, inflammatory bowel disease arthritis, SAPHO [synovitis, acne, pustulosis, hyperostosis, and osteitis] syndrome, chronic recurrent multifocal osteomyelitis), and (c) miscellaneous systemic disorders with arthropathy (relapsing polychondritis, multicentric reticulohistiocytosis, amyloidosis, sarcoidosis, hemophilia). ©RSNA, 2020.

Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that present with unique clinical features but are unified by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. The morphology of cutaneous lesions associated with these disorders is heterogeneous, which renders diagnosis challenging. Moreover, a thorough evaluation is required to exclude diseases that mimic these disorders and to diagnose potential associated infectious, inflammatory, and neoplastic processes. While some neutrophilic dermatoses may resolve spontaneously, most require treatment to achieve remission. Delays in diagnosis and treatment can lead to significant patient morbidity and even mortality. Therapeutic modalities range from systemic corticosteroids to novel biologic agents, and the treatment literature is rapidly expanding. The second article in this continuing medical education series reviews the epidemiology, clinical characteristics, histopathologic features, diagnosis, and management of pyoderma gangrenosum as well as bowel-associated dermatosis-arthritis syndrome and the arthritis-associated neutrophilic dermatoses rheumatoid neutrophilic dermatitis and adult Still disease.

OBJECTIVES In this study, we investigated whether monocyte CD64 (mCD64) expression is correlated with disease activity in patients with adult-onset Still disease (AOSD) and whether it could be used to distinguish between active and inactive disease states. METHODS We reviewed a series of 10 patients with a definite diagnosis of AOSD, recruited from January 2013 to December 2016. We used flow cytometry to quantitatively measure mCD64 expression levels in patients presenting with active and inactive disease states and statistically analyzed the corresponding changes. RESULTS The mean ± SD values of mCD64 expression levels in patients with active and inactive disease states were 77,148.3 ± 39,066.3 and 19,225.8 ± 7006.2 molecules/cell, respectively, indicating significantly higher mCD64 expression in the active state than in the inactive state (p = 0.005). Receiver operating characteristic analysis with a cutoff value of 31,796.0 molecules/cell was applied to distinguish active from inactive disease states; the sensitivity and specificity were both 100%. In these patients, only the mCD64 expression levels changed in parallel with disease activity under tocilizumab treatment; other conventional biomarkers measured showed no changes. CONCLUSIONS Monocyte CD64 expression could be used to clearly distinguish between active and inactive AOSD. Thus, mCD64 could be a promising biomarker for evaluating the disease activity of AOSD, even in patients receiving tocilizumab treatment.

Objective.Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, which can complicate adult-onset Still disease (AOSD). We investigated AOSD clinical features at the time of diagnosis, to assess predictors of MAS occurrence. Further, we analyzed the outcomes of patients with AOSD who experience MAS.Methods.Patients with AOSD admitted to any Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale center were retrospectively analyzed for features typical of AOSD, MAS occurrence, and their survival rate.Results.Of 119 patients with AOSD, 17 experienced MAS (12 at admission and 5 during followup). Twelve patients with MAS at first admission differed from the remaining 107 in prevalence of lymphadenopathy and liver involvement at the time of diagnosis. In addition, serum ferritin levels and systemic score values were significantly higher in the patients presenting with MAS. At the time of diagnosis, the 5 patients who developed MAS differed from the remaining 102 in the prevalence of abdominal pain, and they showed increased systemic score values. In the multivariate analysis, lymphadenopathy (OR 7.22, 95% CI 1.49–34.97, p = 0.014) and abdominal pain (OR 4.36, 95% CI 1.24–15.39, p = 0.022) were predictive of MAS occurrence. Finally, MAS occurrence significantly reduced the survival rate of patients with AOSD (p < 0.0001).Conclusion.MAS occurrence significantly reduced the survival rate in patients with AOSD. Patients with MAS at baseline presented an increased prevalence of lymphadenopathy and liver involvement, as well as high serum ferritin levels and systemic score values. The presence of lymphadenopathy and abdominal pain was associated with MAS occurrence.

Background Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder. A few patients develop organ complications that can be life-threatening. Our objectives were to describe the disease course and phenotype of life-threatening AOSD, including response to therapy and long-term outcome. Methods A multicenter case series of intensive care medicine (ICU) patients with life-threatening AOSD and a systematic literature review. Results Twenty patients were included. ICU admission mostly occurred at disease onset (90%). Disease manifestations included fever (100%), sore throat (65%), skin rash (65%), and arthromyalgia (55%). Serum ferritin was markedly high (median: 29,110 ng/mL). Acute respiratory failure, shock and multiple organ failure occurred in 15 (75%), 10 (50%), and 7 (35%) cases, respectively. Hemophagocytosis was demonstrated in eight cases. Two patients died. Treatment delay was significant. All patients received corticosteroids. Response rate was 50%. As second-line, intravenous immunoglobulins were ineffective. Anakinra was highly effective. After ICU discharge, most patients required additional treatment. Literature analysis included 79 cases of AOSD with organ manifestations, which mainly included reactive hemophagocytic syndrome (42%), acute respiratory failure (34%), and cardiac complications (23%). Response rate to corticosteroids was 68%. Response rates to IVIgs, cyclosporin, and anakinra were 50%, 80%, and 100%, respectively. Conclusions AOSD should be recognized as a rare cause of sepsis mimic in patients with fever of unknown origin admitted to the ICU. The diagnosis relies on a few simple clinical clues. Early intensive treatment may be discussed. IVIgs should be abandoned. Long-term prognosis is favorable. Electronic supplementary material The online version of this article (10.1186/s13054-018-2012-2) contains supplementary material, which is available to authorized users.

Adult-onset Still disease (AOSD) is a systemic inflammatory disease characterized by fever, arthritis and rash. Corticosteroids represent a promising therapeutic option for AOSD; however, some resistant cases require immunosuppressants and biologic agents. We herein report the case of a 29-year-old Japanese man with severe AOSD, accompanied by constrictive pericarditis (CP) and pleuritis. Although 2 courses of steroid pulse and subsequent high-dose of prednisolone and cyclosporine A improved the patient's CP and pleuritis, his fever and inflammatory responses persisted. Tocilizumab (TCZ) was added to his treatment, which resulted in a rapid remission. This case suggests the usefulness of TCZ in the treatment of severe AOSD with CP and pleuritis.

Introduction Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown cause. Its symptoms usually include persistent fever, fugitive salmon-colored rash, arthritis, sore throat (not specific), but it may also lead to internal organs’ involvement, which presents with enlargement of the liver and spleen, swollen lymph nodes, carditis or pleuritis – potentially life-threatening complications. In rare cases, AOSD can cause aseptic meningitis or/and encephalitis. Case presentation We report a case of 31-year-old male patient, who was referred to neurological department for extending diagnostics of frontal lobes lesions with involvement of adjacent meninges. Abnormalities have been revealed in brain MRI, which was performed due to persistent headaches, visual disturbances, fever, fatigue and cognitive decline. Wide differential diagnosis was performed including laboratory findings, contrast enhanced MRI, MR spectroscopy, flow cytometry and finally brain biopsy to exclude neoplastic or infectious origin. Final diagnosis of autoimmune meningoencephalitis in adult-onset Still disease has been made. Conclusion Adult-onset Still disease is a rare cause of inflammatory changes in central nervous system, which if diagnosed, may be treated successfully with steroids (commonly available agent), intravenous immunoglobulins or more specific immunomodulating regiments.

Adult-onset Still disease (AOSD) is a rare autoinflammatory syndrome characterized by recurring fevers, arthralgia, and consistent laboratory abnormalities that include leukocytosis and hyperferritinemia. Skin findings accompany the disease in nearly 90% of the cases. Early reports described evanescent, pruritic, salmon-pink or urticarial lesions, referred to as the typical eruption of AOSD. Histopathologic findings consist of superficial perivascular dermatitis with varying number of interstitial neutrophils. Later reports described a more persistent rash that tended to be photodistributed, hyperpigmented, often in a linear configuration, sometimes in a rippled pattern, referred to as the atypical eruption of AOSD. The presence of individual necrotic keratinocytes in the upper spinous layer has been the consistent histopathologic finding. The persistent rash may not represent an atypical presentation of AOSD as recent reports indicate a high prevalence of the rash. Emerging data also suggest that patients with persistent eruption have a worse prognosis. The recognition of the clinical and histopathological findings of skin eruptions of AOSD may facilitate an earlier diagnosis, potentially improving disease outcome. Herein, clinical and histopathological features of cutaneous manifestation of AOSD in 2 Asian women are highlighted accompanied by a relevant review of the disease.

Objective.To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with adult-onset Still disease (AOSD).Methods.We performed a retrospective analysis of patients with AOSD with fever who were admitted to Severance Hospital between 2005 and 2016. The patients with AOSD were evaluated for MAS using the 2016 classification criteria for MAS. Clinical features, laboratory findings, and overall survival were analyzed. Logistic regression analysis was used to evaluate the factors associated with in-hospital mortality.Results.Among 64 patients with AOSD, 36 (56.3%) were classified as having MAS. The overall survival rate was significantly lower in patients with MAS than in those without (67% vs 100%, p < 0.001). Multivariate analysis showed that a low erythrocyte sedimentation rate, a low albumin level, an increase in ferritin of over 2 folds, and the development of MAS on admission were significantly associated with mortality in patients with AOSD.Conclusion.The 2016 EULAR/ACR/PRINTO classification criteria for MAS are potentially useful for the identification of patients with AOSD at high risk for a poor outcome. Febrile patients with AOSD should be monitored with the 2016 classification criteria for MAS in the early diagnosis and proper treatment of MAS.

Background Adult onset Still’s disease (AOSD) is a rare condition characterized by four cardinal signs: fever, arthralgia/arthritis, rash, and neutrophilic leukocytosis. The absence of specific criteria is currently compensated by pathophysiological advances, leading to the introduction of a promising new therapeutic arsenal represented by biotherapies. Objectives The aim of this study was to compare the profiles of this disease in our latitudes with data from foreign literature; and to study the most recent data concerning the pathophysiology and treatment of this disease. Methods We conducted a retrospective study of hospital files from January 1993 to October 2018. The diagnosis is retained according to Yamaguchi criteria. Analysis was performed using IPSS. Results 17 patients were selected. The sex ratio was 0.54, and the average age was 31.5 years. At the time of diagnosis, fever was found in 94% of cases, arthralgia/arthritis in 100% of cases, and rash in 65% of cases. Concerning rheumatic features, the most frequently affected joints were: wrists (87%), knees (73%), shoulders (67%), proximal interphalangeal (62%), elbows and ankles (60%), hips (47%). The involvement of the hands is dominated by the proximal interphalangeal and metacarpophalangeal (45%). Distal interphalangeal involvement is observed in 23% of cases. Cervical spine involvement is noted in 50% of cases. Biologically, 88% of patients had neutrophilic leukocytosis. Ferritinemia was increased in 79% of patients. The glycosylated fraction of ferritin was measured at diagnosis in 47% of patients, of whom 63% had a fraction ≤ 20%. Elevated transaminases were found in 47% of cases. Therapeutically, 100% of patients received corticosteroids; 65% methotrexate; and 29% biotherapies (tocilizumab, anakinra, infliximab, adalimumab). The evolutionary profile was exploitable in 13 patients, of whom 10 were in complete remission. Conclusion Our data are consistent with those in the literature. Some peculiarities emerge concerning the articular features. From 3 series of literature and ours, totaling 135 cases, it appears that the most frequent locations are: wrist (82%), knees (70%), elbows (46%), ankles (45%), proximal interphalangeal (44%), shoulders (42%) and metacarpophalangeal (42%). The pathogenesis of AOSD seems to lie at the crossroads between auto-inflammatory diseases and lymphohistiocyte activation, resulting from a multifactorial predisposition. The central role of innate immunity, the clinical presentation, and the efficacy of blocking the IL-1 pathway comparable to that observed in “monogenic inflammasomopathies” justify the reclassification of AOSD among polygenic auto-inflammatory syndromes. References [1] Georgin-Lavialle S, et al. [Clinical overview of auto-inflammatory diseases]. Rev Med Interne. 2018;39(4):214-32. [2] Gerfaud-Valentin M, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine (Baltimore). 2014;93(2):91-9. [3] Wouters JM, et al. Adult-onset Still’s disease; clinical and laboratory features, treatment and progress of 45 cases. Q J Med. 1986;61(235):1055-65. [4] Pouchot J, et al. Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore). 1991;70(2):118-36. [5] Ben Taarit C, et al.[Adult Still’s disease: study of a series of 11 cases]. J Mal Vasc. 2002;27(1):31-5. Disclosure of Interests None declared